Fibrotic disorders affect many organ systems, including heart, blood vessels, kidney, liver and lung. An estimated 45% of deaths in the United States are attributed to disorders that are characterized by varying degrees of fibrosis. This alarming statistic is often underappreciated since the ‘cause of death’ is often end-stage organ failure, although in many cases organ failure is attributed to progressive fibrosis.
The most severe and deadly fibrotic lung disease is idiopathic pulmonary fibrosis (IPF), characterized by progressive scar tissue formation and irreversible destruction of the lung parenchyma, resulting in gas-exchange abnormalities and ultimately respiratory failure. IPF is widely regarded as a disease of aging, as it disproportionately affects the elderly population with a mean age of 66 years at the time of diagnosis. IPF is associated with high morbidity and mortality with a median survival rate of less than three years. Further, the survival rate for IPF patients markedly decreases with age.
Although two drugs have recently gained FDA-approval for IPF, no drug treatment has been shown to definitively improve quality of life or survival for IPF patients. The current drugs only moderately slow the progression of lung decline. There are no available therapies that can ‘reverse’ fibrosis.
Therefore, there is a clear need for more effective treatments for IPF and other fibrotic diseases in order to improve the patient experience and outcomes.